Is there a difference in airway clearance practices
between children with non cystic fibrosis bronchiectasis and cystic
fibrosis?

Abstract:

Studies have investigated airway clearance techniques in cystic
fibrosis (CF) but few exist for non-CF bronchiectasis particularly in
children. The aim of this study is to compare airway clearance practices
in New Zealand children with CF and non-CF bronchiectasis. The study was
conducted in the context of a clinical quality assurance audit of a
hospital clinic service. Children and their families who live in the
Greater Auckland area and who attend tertiary respiratory clinics were
interviewed by a physiotherapist, who completed a questionnaire on
frequency of cough, amount and type of airway clearance performed.
Demographic data, age at diagnosis, duration of disease and the New
Zealand Deprivation Index were noted. Children were grouped according to
age-appropriate airway clearance techniques. One hundred and six
questionnaires were completed (81 bronchiectasis; 25 CF). Significant
differences were demonstrated for ethnicity, deprivation score and
duration of disease between the groups. In the 9-17 year olds 75% used a
positive expiratory pressure device (23/35 bronchiectasis; 13/13 CF). In
the 0-4 year olds 60% performed modified postural drainage (13/25
bronchiectasis; 5/5 CF). When well, children with CF did more regular
airway clearance than those with bronchiectasis (p=0.0004). Both groups
increased the amount of airway clearance when unwell (p=>0.05).
Similar numbers (<8%) reported doing no airway clearance techniques.
The implications of the findings for paediatric physiotherapy practice
in New Zealand are discussed.

Reported adherence to treatments in CF disease has varied depending
on the specific treatment studied. For example, in children there is
16-20% adherence recorded for dietary supplementation but up to 67-84%
for dornase alpha (Modi et al 2006). Chest physiotherapy is at the lower
end of adherence (29-55%) when measured in studies of CF adults (Abbott
et al 1994, Abbott et al 1996, Conway et al 1996, Kettler et al 2002,
White et al 2007) with only one study recording adherence as high as 70%
(White et al 2007) and reports of between 40-47% in children (Modi et al
2006). In addition, the adherence recorded varies depending on whether
it is ascertained by parental report, child report, daily diary,
pharmacy refills or electronic monitoring (usually decreasing across
these parameters) (Modi et al 2006). Although there is much written
about ACTs in the paediatric CF population there is very little on
paediatric non-CF BX (Jones and Rowe 2007, O'Neill et al 2002) and
results cannot necessarily be extrapolated between these groups.

There are a number of reasons why regular and effective airway
clearance practices may be more important for New Zealand children with
non-CF BX than other populations:

* The prevalence and incidence of non-CF BX has been reported to be
very high (Twiss et al 2005);

* The disease seen is severe and widespread based on lung function
and number of lobes affected (Edwards et al 2003, Edwards et al 2004a);

* A comparative study of exercise in children with CF and non-CF BX
in the UK has shown significant exercise limitation in children with
non-CF BX (Edwards et al 2003, Edwards et al 2004a).

In addition a previous unpublished clinical audit of 20 children
with non-CF BX during establishment of the BX clinic at Starship
Children's Hospital in Auckland in 1999 (conducted by GN, CT)
showed 84% used head down postural drainage and active cycle of
breathing technique with 21% intermittently compliant and 48%
non-compliant. Thirty five percent felt this was of little or no use,
with 75% either decreasing or not altering treatment frequency when the
child was unwell. Thirty-five percent of the children reported being
either 'not usually happy' or 'never happy' with
their ACT, alarmingly often giving 'pain' or
'discomfort' as reasons for this. In response, the service
developed educational resources (diagram, pictures and video) including
a written action plan for use during stability and exacerbations. Each
family was linked with an experienced respiratory physiotherapist for
visits in clinic and at home. The CF service already had this
educational and physiotherapy support, and families appeared to have a
better understanding of the disease.

The aims of this current study were to use a questionnaire to
compare airway clearance practices in children attending the BX and CF
clinics at Starship Children's Hospital, a tertiary paediatric
hospital, to examine how demographic data related to patterns of ACT use
and to review whether the educational input had altered levels of
adherence in our clinic.

METHODS

Only families living in the Greater Auckland Region and who
attended either the Starship CF or non-CF BX clinics were invited to
participate over a 6 month period from May 2006. During the time the
audit was undertaken 25 of the potential 50 children who attended the
Starship CF clinic, and 106 of the potential 150 children who attended
the Starship Bx clinic were approached. None of the families declined to
join the study. Following verbal consent, a questionnaire was completed
with each family by one of three respiratory physiotherapists (SB, LH,
JH) in the outpatient clinic, on the ward or in the child's home.
All participants spoke English although an interpreter was available if
necessary. Respondents (usually the mother, and the child if old enough)
were all asked the same questions relating to the amount of daily
coughing (as a crude measure of symptom severity), number of sessions
and type of ACTs performed when well and unwell. School aged children
were also asked the number of formal exercise sessions per week of more
than 30 minutes duration. Demographic data, age at diagnosis, duration
of disease and the New Zealand Deprivation 2001 Index (NZDep2001)
(Salmond and Crampton 2002) were recorded. The index ranges from score 1
(10% least deprived nationally) to score 10 (10% most deprived).

The questionnaire results were interpreted in three age brackets
(0-4, 5-8 and 9-18 years) corresponding to age-appropriate
recommendations on ACTs and increasing self-responsibility. Statistical
advice and analyses were undertaken by a statistician (PR) with the
Stats Direct (www. statsdirect.com) MS Excel add-in software. The
children's characteristics were expressed as means with standard
deviations or medians with ranges depending upon whether they were
normally distributed as ascertained by the Kolmogorov-Smirnov test.
Fishers Exact statistic was used to test for differences in the
distribution of responses between the Bx and CF cohorts, and between the
age categories. A p value less than 0.05 was taken to be statistically
significant. Verbal consent was obtained from the parent and child if
old enough. The survey was designed as a clinical quality assurance
audit and although discussed with the local ethics committee formal
approval was not required.

RESULTS

One hundred and six questionnaires were completed over the 6 month
study period--81/150 non-CF BX (54% of the clinic population) and 25/50
CF children (50% of the clinic population) with no significant
differences seen between age or gender (see Table 1). There was a
significant difference in ethnicity with 85% of children with BX from
Maori or Pacific Island peoples and 72% of children with CF from
European descent (p<0.0001). Significant differences were seen in the
deprivation scores resulting in 75% of children with BX compared to 12%
of children with CF living in the 30% most deprived areas (decile 8 and
above) of New Zealand. The children with CF were diagnosed significantly
younger and had a longer duration of lung disease (see Table 1).

There was no statistical difference between the groups with amounts
of cough when well. In the BX cohort: 20 (25%) reported no cough, 24
(30%) reported coughing with exercise only and 24 (30%) coughed both day
and night. Of the CF cohort: 11 (44%) reported no coughing, 7 (28%)
reported coughing during exercise, and 5 (20%) coughed both day and
night. These differences were not statistically significant. There was a
statistical difference in reported cough between the groups when unwell
(p=0.01) with all the BX group reporting significant cough, 5 (6%)
during the day and 76 (94%) both day and night, compared to the CF group
with 2 (8%) denying coughing even when unwell, 4 (16%) reporting
coughing during the day or with exercise and 19 (76%) coughing day and
night.

When well, 30 (37%) of children with BX did their ACT at least
daily compared to 20 (80%) of children with CF (p=0.0004). When unwell,
the difference was less significant with 63 (78%) of BX and 22 (88%) of
CF patients doing their ACT at least once a day (p = ns). There was no
significant difference between the groups in those doing no ACT (6 (7%)
with BX, 1 (4%) with CF, p= >0.05). Reasons given why regular ACT
were not undertaken included nearly half saying it was 'hard for
them to do' (46% with BX, 44% with CF), they 'couldn't be
bothered' (38% with BX, 64% with CF), it 'hurt' (14% with
BX, 27% with CF), or because of a lack of time (43% with BX, 9% with
CF).

In the 9-18 year age group 28 (38%) of those with BX and 14 (54%)
of those with CF reported having problems with ACTs. Despite these
difficulties 65 (80%) and 11 (44%) of children with BX or CF
respectively felt that doing ACTs made them feel better, which was
significantly different between the groups (p=0.0008). In the 9-18 year
olds 75% used a Positive Expiratory Pressure device (PEP) (23/35
bronchiectasis; 13/13 CF) (see Table 2). In the 0-4 year olds 60%
performed modified postural drainage (13/25 bronchiectasis; 5/5 CF) (see
Table 2). One hundred percent of the CF and 81% of the BX children did
their ACT at home (p>0.05), 14% had treatment elsewhere such as at
relatives' homes and 5% were treated at school.

In the 5-8 year old children, 71% of the BX and 72% of the CF
played sport at least once a week (p=>0.05) and 19% and 43%
respectively played organised sport at least every day/week (p=0.03). In
the older children and young people (9-18 years), 72% of the BX and 100%
of the CF teenagers played sport at least once a week (p=0.03) and 28%
and 62% respectively played organised sport at least every day per week
(p=0.03).

DISCUSSION

Our practice (Edwards et al 2004b), in keeping with others (Chang
et al 2002, Fibrosis 2002, Lannefors et al 2004, Wallis and Prasad
1999), is to recommend regular ACT and exercise programmes for all
children with CF and non-CF BX, despite minimal supporting research
evidence (Jones and Rowe 2007). As far as we are aware this is the first
report to compare self-reported adherence to ACTs and exercise in these
groups of children.

Three decades ago, a review of 537 studies showed the mean
adherence to long term therapies was 54% (range 41-61%) (Sackett and
Snow 1979). In our study, the adherence to recommendations on ACT in Bx
was well below this level when well (37%) but above average when unwell
(78%). However, the adherence in our study for CF was far above average
when well or unwell. The earlier diagnosis and longevity of disease in
CF may have had a beneficial effect on adherence. Regular chest
physiotherapy, including airway clearance training from an early age may
facilitate acceptance and enable caregivers to develop these skills,
while introducing new treatments to older children can be fraught with
difficulties (Patterson et al 2005, White et al 2007). This is different
from the finding that adherence decreases as duration and complexity of
treatment increases (Kettler et al 2002). Resourcing for clinics are
also different with the CF society providing the clinic with a full time
social worker and 16 hours of physiotherapist time in the community,
while there is no BX family support group, a part time physiotherapist,
no dedicated social worker and no current cultural support worker. A
study of adults with CF conducted through the UK CF Trust showed
consultation with a physiotherapist was positively associated with
increased adherence (Myers and Horn 2006).

A similar, small percentage of both groups (<8%) undertook no
ACTs. Although reasons given for not doing regular treatments varied, in
the main they were 'could not be bothered' and 'lack of
time'. These are consistent with other studies reporting interview
data in teenage and adult CF groups with 61%-80% giving these same two
reasons (Abbott et al 1996, Conway et al 1996, Fielding and Duff 1999,
Myers and Horn 2006, White et al 2007). Other responses from the studies
included 26-45% felt they were well without performing ACT (Abbott et al
1996, Conway et al 1996, White et al 2007), 13-37% felt their disease
was less severe than others (Abbott et al 1996, White et al 2007),
10%-53% were too tired (Modi et al 2006, White et al 2007), 35% said it
interrupted their social life (Abbott et al 1996) and 22-35% simply
forgot (Abbott et al 1996, White et al 2007). In one study respondents
gave twice as many reasons for omitting ACT than for any other treatment
studied (Conway et al 1996). The lack of time reported in the BX cohort
may be due to the number of families attending BX clinic with higher
numbers of children in the family.

Studies assessing adherence in CF show that perceived severity or
increased 'worry' about their disease was associated with
improved adherence (Abbott et al 1996, Gudas et al 1991). Patients who
report an immediate improvement in symptoms after ACT have also been
shown to have improved compliance (Lavery et al 2007). This may account
for the increase in compliance seen in the BX group when they became
unwell. However, while more of the BX cohort compared to the CF reported
feeling better after performing their ACT, the BX group did less regular
treatment than the CF group.

Overall, the BX group continued to have lower adherence rates than
their CF counterparts despite being managed by the same medical team.
The Chronic Care Model endorsed by the World Health Organisation
suggests that patient outcomes can be improved by proactive healthcare
providers informing and activating patients (Lavery et al 2007). In a
study of adult BX patients perceived obstacles to self-management
included lack of information and confidence (Lavery et al 2007). The
significant cultural differences between the families attending the BX
or CF clinics (Twiss et al 2005) are likely to impact on understanding
the disease and treatment. In our clinic in 1999, 15/20 (75%) of BX
families either only maintained or actually decreased ACT during an
acute exacerbation. In this study 63/81 (78%) did at least daily
sessions when unwell following extra educational input. While this may
be seen as an improvement it is still suboptimal.

Exercise also improves both physical and mental well-being and has
been shown to improve aerobic capacity, endurance exercise capacity and
inspiratory muscle strength in both BX and CF (Barak et al 2005,
Lannefors et al 2004) with 17-38% admitting a preference for exercise
than doing ACT (Abbott et al 1994, Conway et al 1996, Myers and Horn
2006, White et al 2007). In our survey, children with BX undertook less
sport than children with CF, perhaps because of the socioeconomic
differences seen and/or the severity of lung disease with increased
cough reported in the BX group. Formal exercise testing has yet to be
reported for New Zealand children with BX, but the literature suggests
significant exercise limitation in comparison with normal children and
with CF (Edwards et al 2004a).

The type of ACTs best suited to individual respiratory patients
continues to be debated, with the choice decided by culture, tradition,
economics and geographical influences (O'Neill et al 2002),(Barak
et al 2005, Homnick 2007, Lavery et al 2007, Patterson et al 2005,
Thompson et al 2002). A 2002 survey of UK BX clinics (predominantly
adult) showed the majority used active cycle of breathing technique
(91.5%) and/or postural drainage (75.6%) (O'Neill et al 2002). In
contrast, in our study 75% of the patients over the age of 9 years used
PEP. They found it more discrete, more enjoyable and more flexible (e.g.
able to do in car, at the marae, etc). Caregivers also reported finding
PEP easier to monitor and they did not have to be actively involved in
the treatment. This confirms previous findings that the least difficult
programme and those requiring the least alteration to lifestyle will
tend to be favoured (Barak et al 2005, Gudas et al 1991, Homnick 2007,
Thompson et al 2002). We have also found PEP easier to teach in school
settings, which are the only treatment session administered to some of
the patients. While other devices such as Flutter and Acapella are
currently used by some of the children in both clinics at the time the
study was undertaken none of the patients used either of these devices.
In the 0-4 year age group, 60% were treated without a head down tip
subsequent to demonstration that the previous head down recommendation
caused increased gastro-oesophageal reflux in children with CF (Button
et al 2003, Button et al 2004, Lannefors et al 2004).

This survey has a number of weaknesses and the findings cannot be
extrapolated to other populations or groups. In this report of a quality
assurance audit, only approximately 50% of the clinic populations were
able to be surveyed within the study period but none approached
declined. There were fewer children with CF within the catchment area
which led to unequal numbers between the groups. The questions asked
were based on standard questions asked at clinic visits by our
physiotherapy team; however no measures of reliability and validity were
undertaken. Three interviewers (all physiotherapists) were involved
rather than one or repeat interviews by 2 people.

This survey only assessed adherence to the recommended
physiotherapy programme by direct interview; this may well have led to
over-reporting of adherence by the families. One study has showed
significant differences in adherence when reported by parent, child,
daily diary record or measured by electronic devices (Modi et al 2006).
Specifically, ACT frequency adherence decreased from 74% by parent
report to 67% by child report to 51% by diary record (Modi et al 2006).
Other studies used a scale or a score for interview data to assess
adherence (Abbott et al 1994, Conway et al 1996).

While these risks of bias created could have been addressed by the
questionnaire being postal or completed independently, this may have
reduced the response rate. Finally cough was the only parameter used as
a crude measure of severity of disease rather than radiological, lung
function or formal exercise testing, and severity of disease may affect
adherence as mentioned above (Abbott et al 1996, Passero et al 1981).
The primary study aim was not to assess severity of disease but to
investigate airway clearance practices. Despite these weaknesses the
study has a number of important implications for paediatric respiratory
and physiotherapy practice in New Zealand.

CONCLUSIONS

In this survey, undertaken as part of a quality assurance audit,
the Bx group showed less adherence to the treatment recommendations than
their CF counterparts. The significant differences documented regarding
ethnicity, age of diagnosis, disease duration and socioeconomic status
may provide some explanation. We acknowledge that, as this survey was
undertaken in the clinics of only one hospital, the findings may not be
generalisible to other centres. If we could improve some of the
socioeconomic hurdles and provide equal resourcing for the two clinics,
plus improve access to culturally appropriate education around the
disease and treatment, the differences in adherence between these groups
might not be so great.

Key Points

* Current best practice is to recommend airway clearance techniques
which are individualized and undertaken regularly for all children with
cystic fibrosis and bronchiectasis.

* This is the first report to compare self-reported adherence to
prescribed airway clearance techniques in children with cystic fibrosis
and non-CF bronchiectasis.

* New Zealand children with non-CF bronchiectasis showed less
adherence to treatment recommendations than their CF counterparts.

* In New Zealand improving some of the socioeconomic hurdles and
providing equal resourcing may improve adherence in these children.